Prevalence, mutation spectrum, and cardiac phenotype of the Jervell and Lange-Nielsen syndrome in Sweden

EP Europace, 05/16/2012

Founder effects could explain 83% of the Swedish jervell and lange–nielsen syndrome (JLNS) mutation spectrum and probably contribute to the high JLNS prevalence found in preadolescent Swedish children. Due to the severe cardiac phenotype in JLNS, the importance of stringent β–blocker therapy and compliance, and consideration of ICD implantation in the case of therapy failure is stressed.

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